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Late semester 2 exams > Immunodeficiencies / Autoimmune processes > Flashcards

Immunodeficiencies / Autoimmune processes Flashcards

(65 cards)

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1
Q

Asplenia

A

Encapsulated extracellular bacteria (not removed from blood)

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2
Q

C3 deficiency

A

Recurrent G-, encapsulated bacterial infection (for all alternative complement pathway disruptions)

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3
Q

Asplenia

A

Encapsulated extracellular bacteria

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4
Q

C3 deficiency

A

Recurrent G- bacterial infection

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5
Q

Factor I deficiency

A

Absence of factor I –> depletion of C3

Encapsulated bacteria

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6
Q

Hereditary angioneurotic edema

A

Deficiency of C1 inhibitors –> depletion of C4, C2

Recurrent episodes of edema skin, larynx, GI, & UG tracts

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7
Q

Paroxysmal nocturnal hemoglobinuria

A

Deficient expression of DAF & CD59 –> intravascular hemolysis
(X-linked)

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8
Q

Autoimmune hemolytic anemia

A

Rh blood group antigens, I antigen; Destruction of RBCs via complement, phagocytosis –> anemia (2)

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9
Q

Autoimmune thrombocytopenia purpura

A

Platelet integrins gpIIb:IIIa –> abnormal bleeding (2)

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10
Q

Goodpasture’s syndrome

A

Basement membrane, collagen type IV –> Glomerulonephritis, pulmonary hemorrhage (2)

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11
Q

Pemphigus vulgaris

A

Epidermal cadherin –> Skin blisters (2)

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12
Q

Pemphigus foliaceus

A

Desmoglein –> mild skin blistering (2)

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13
Q

Acute rheumatic fever

A

Group A Streptococcal cell wall antigens (Abs cross react with endocardium) –> Arthritis, myocarditis, heart valve scarring (2)

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14
Q

Grave’s disease

A

TSH receptor (agonist) –> hyperthyroidism (2); IgGs may be transmitted to fetus through placenta

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15
Q

Myasthenia gravis

A

ACh receptors –> Progressive weakness (2)

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16
Q

T2 diabetes

A

Insulin receptor (antagonist) –> hyperglycemia, ketoacidosis (2)

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17
Q

Hypoglycemia

A

Insulin receptor (agonist) –> Hypoglycemia (2)

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18
Q

Subacute bacterial endocarditis

A

Bacterial antigens –> soluble immune complexes –> glomerulonephritis (3)

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19
Q

Cryoglobulinemia

A

RF IgG complexes –> Vasculitis (3)

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20
Q

Systemic lupus erythemaosus

A

DNA, histones, ribosomes –> Glomerulonephritis, vasculitis, arthritis (3)

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21
Q

T1 diabetes

A

Pancreatic beta-cell Ag –> beta-cell destruction (4)

Coxsackie A&B virus may trigger

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22
Q

RA

A

Unknown synovial joint Ag –> Joint inflammation, destruction (4)

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23
Q

MS

A

Myelin basic protein –> Brain degeneration, paralysis (4)

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24
Q

APECED (Autoimmune PolyEndocrinology Candidiasis Ectodermal Dystrophy)

A

Mutations in AIRE (AutoImmune REgulator) –> defective thymic deletion of autoreactive T cells (TECs can’t express peripheral Ags) –> wide ranging autoimmune rxns

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25
IPEX (Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked syndrome)
Mutations in transcription regulator of FOXP3 --> Treg cells aren't produced --> High, autoreactive B, T IRs
26
HLA allotypes: | B27, A29, DQ6
Associated w/: B27 - Ankylosing spondylitis A29 - Birdshot chorioretinopathy DQ6 - Narcolepsy, MS, (diminished risk of T1 diabetes)
27
Ciliac's
Wheat flour gluten modifies tissue transglutaminase --> Anti-transglutaminase CD4+/B cells
28
Borrelia burgdorferi
Associated with chronic autoimmune arthritis
29
Campylobacter jejuni
Associated with reactive arthritis, GBS
30
Chlamydia trachomatis
Associated with Reiter's syndrome (arthritis)
31
Hashimoto's thyroiditis
Hypothyroidism, autoimmune destruction, antagonists of TSH receptors
32
Crohn's disease
Abnormal mucosal T cell regulation --> Granulomatous rxn
33
Pernicious anemia
Anti-parietal cell/anti-intrinsic factor Abs --> impaired vit B12 absorption from lack of intrinsic factor in gastric secretions
34
Autoimmune chronic active hepatitis
Liver cells express MHC II --> Anti-liver cell Abs
35
Deficiencies of any of C5-9
Lack of complement-mediated lysis --> Neisseria susceptibility
36
NEMO deficiency
Impaired NFkB activation --> Chronic bacterial/viral infections
37
Chronic granulomatous disease
Defective NADPH oxidase in neutrophils --> Chronic bacterial/fungal infections
38
MBL (mannose-binding lectin) deficiency
Neisseria meningitidis --> Meningitis
39
NK-cell deficiency
GATA2 gene impaired --> No NK cells --> Severe herpesvirus infections
40
Hyper-IgM deficiency
CD40/CD40L impaired --> No isotype switching/somatic hypermutation in B cells --> Extracellular bacterial/fungal infections
41
IgG2 deficiency
Susceptible to encapsulated bacteria
42
SCID
Various genes affected (ADA, PNP, common gamma chain in IL receptors, repair mechs.) --> B & T cell fxn absent --> All infxns
43
Omenn syndrome
RAG1/2 --> Impaired VDJ recombination --> Can't form TCRs/BCRs properly --> All infxns
44
X-linked agammaglobulinemia
BTK doesn't signal --> B cells blocked at pro-B stage --> Recurrent bacterial infxns
45
Complete DiGeorge syndrome
Absence of thymus, T cells --> All infxns
46
ZAP-70 deficiency
T cells cannot signal through TCRs --> All infxns
47
Selective IgA deficiency
No major susceptibility, most common immunodeficiency
48
DiGeorge's syndrome
Thymic aplasia --> General infection susceptibility
49
MHC I deficiency
No CD8+ T cells --> Intracellular infection susceptibility
50
MHC II deficiency
No CD4+ T cells --> SCID
51
Graft-Vs-Host disease
Donor T cells in bone marrow grafts attack tissues of IC'd hosts --> allogenic IR, inflammatory state; Immunosuppressive drugs, deplete grafts of T cells
52
Interferon-G deficiency
Macrophages do not activate --> No granulomas, susceptible to intracellular pathogens
53
Serum sickness
Introduced proteins or drugs induce formation of Abs targeting them, esp. w/multiple exposures --> immune complexes form (Arthus rxn)
54
Atopic dermatitis
Eczema associated w/Type I hypersensitivity, common in young patients
55
Adenosine deaminase deficiency
Decreased DNA synthesis --> Low T & B cell counts --> SCID
56
Selective IgA deficiency
Slight susceptibility to mucosal pathogens, most common immunodeficiency
57
Adenosine deaminase deficiency
Decreased DNA synthesis --> Low T & B cell counts --> SCID; Need bone marrow transplant
58
Leukocyte adhesion deficiency
Deficient integrin B2/CD18/LFA-1/CR3 in neutrophils --> Defective migration to inflammation sites --> Chronic bacterial infxns
59
Toxic shock syndrome
Superantigen TSST from Staph, Strep --> Polyclonal CD4+ activation (~20% of total) --> Cytokine storm --> Organ failure
60
Leukocyte adhesion deficiency
Deficient integrin B2/CD18/LFA-1/CR3 in neutrophils/macrophages --> Defective migration to inflammation sites --> Chronic bacterial infxns
61
Toxic shock syndrome
Superantigen TSST from Staph, Strep --> Polyclonal CD4+ activation (~20% of total) --> Cytokine storm --> Organ failure
62
G6PD & Myeloperoxidase deficiency
Defective phagocytes can't kill pathogens --> Chronic bacterial/fungal infxns
63
Chediak-Higashi syndrome
Lysosoal trafficking regulator protein defective --> Defective phagocytosis/phagolysosome formation --> Persistent bacterial infxns, granulomas
64
HIV
Depletion of CD4+ T cells --> IC | Opp. infxns include Toxoplasma, Mycobacterium, Cryptococcus, Pneumocystis, Candida, Herpesviruses
65
Radiation, chemotherapy, & metastases to bone marrow
Inhibition of leukocyte developmet --> IC

Late semester 2 exams (2 decks)

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